Langerhans Cell Histiocytosis beyond Childhood

Abstract

Introduction: Langerhans cell histiocytosis (LCH), is an uncommon hematological disorder affecting infants and young children, with male predominance. It is considered a rare disease, and its real incidence is unknown. With an annual incidence of approximately 2 - 5 cases per million individuals, it occurrs more frequently in children (4 - 8 per million per year) than in adults (1 - 2 per million per year). The disease hallmarks are round and have characteristic “coffee-bean” cleaved nuclei and eosinophilic cytoplasm. Case Pr esentation: We describe the case of a 19-year-old female who is known as the case of Langerhans cell histiocytosis. Presented to our department at the age of 11, when she started to develop skin rash in her chest with redness and papules, which then became an ulcer after that started in her scalp. Microscopic description of biopsy sections shows a cellular infiltrate composed predominantly of large mononuclear cells with abundant pale eosinophilic cytoplasm and irregular, grooved (“coffee-bean”) nuclei. The background contains a mixed inflammatory infiltrate rich in eosinophils, along with scattered lymphocytes, plasma cells, and occasional multinucleated giant cells. Conclusions: The definitive diagnostic approach for Langerhans Cell Histiocytosis (LCH) is histopathological examination. Once diagnosed, the treatment plan is determined based on the severity of the disease. In adults with LCH, treatment strategies vary depending on the organs involved, the clinical stage of the disease, the patient’s age, and the extent of organ dysfunction.